Craneosinostosis, una perspectiva pediátrica

V. Fernández, H. Chica, R. Goycoolea
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Abstract

Craniosynostosis is defined as the premature fusion of one or more skull sutures, characterized by an abnormal shape of the head. It is a rare condition but should be recognized and timely referred to Neurosurgery in order to prevent complications. The objective of this review is to describe the most frequent clinical and genetic characteristics of this pathology, its classification according to the shape of the skull, and the most characteristic signs to achieve timely recognition. A search for scientific articles in Pubmed, Scielo, and EMBASE databases was performed using the terms craniosynostosis, plagiocephaly, scaphocephaly, and brachycephaly. We selected articles in Spanish and English that described the characteristics of the pathology and about its management, choosing systematic reviews or recommendations from scientific societies when available. Craniosynostosis may occur in isolation or associated with other deformities. Its classification depends on the affected suture(s), leading to the characteristic shape of the skull and the presence of other malformations. This condition is usually diagnosed and referred late, which is associated with complications such as intracranial hypertension and impaired brain development. Early surgery has less comorbidity and better esthetic results. In conclusion, the abnormal shape of the skull must raise the suspicion of craniosynostosis, even if it occurs in isolation. Surgical management before one year of life is associated with a better prognosis.
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颅缝成形术,儿科视角
颅骨缝合术是指一个或多个颅骨缝合线过早融合,其特征是头部形状异常。这是一种罕见的情况,但应予以识别并及时转诊至神经外科,以防止并发症。这篇综述的目的是描述这种病理最常见的临床和遗传特征,根据颅骨形状对其进行分类,以及最具特征的体征,以实现及时识别。在Pubmed、Scielo和EMBASE数据库中搜索科学文章,使用术语颅骨缝合、斜头畸形、舟状脑和短头畸形。我们选择了西班牙语和英语的文章,描述了病理学的特征及其管理,并选择了科学学会的系统综述或建议。颅骨骨质疏松症可能单独发生或与其他畸形相关。其分类取决于受影响的缝合线,导致颅骨的特征形状和其他畸形的存在。这种情况通常诊断和转诊较晚,与颅内高压和大脑发育受损等并发症有关。早期手术合并症较少,美观效果较好。总之,颅骨的异常形状一定会引起人们对颅骨缝合症的怀疑,即使它是孤立发生的。在生命一年之前进行手术治疗与更好的预后相关。
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